This is an open label sudy to examine the effect of exendin-(9-39)on glucose requirements to maintain euglycemia in infants with congenital hyperinsulinism unresponsive to medical therapy and to determine therapeutic plasma levels,plasma half life and pharmacokinetics of exendin-(9-39)

Eligibility

Ages Eligible for Study:

up to 12 Months (Child)

Genders Eligible for Study:

Both

Accepts Healthy Volunteers:

No

Criteria

Inclusion Criteria:

Confirmed diagnosis of congenital hyperinsulinism (based on clinical criteria: insulin, beta hydroxybutyrate, and/or free fatty acid plasma levels at the time of hypoglycemia, and/or glycemic response to glucagon at the time of hypoglycemia)

Age: from birth to 12 months

Failure to respond to diazoxide (defined as the failure to maintain blood glucose and octreotide (defined as the failure to maintain blood glucose ≥ 70 mg/dL without supraphysiologic rates of glucose infusion> 4-5 mg/Kg/min)

Current therapy at the time of initiation of study procedures with medications that affect glucose metabolism, such as high dose glucocorticoids, ß-agonists, glucagon, diazoxide and octreotide. Subjects will be eligible to participate 4 hours after glucagon is discontinued, 24 hours after the last dose of octreotide and 72 hours after last dose of diazoxide,

Subjects with suspected Beckwith-Wiedemann syndrome or other syndromic forms of congenital hyperinsulinism.

Contacts and Locations

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Please refer to this study by its ClinicalTrials.gov identifier: NCT00835328